This report describes the case of a full-term gestational female with a prenatal diagnosis of pulmonary atresia with intact ventricular septum. Cardiac ultrasound at birth confirmed the diagnosis with no evidence of coronary artery fistulas. The patient died 6 hours after a central aortic to pulmonary artery shunt had been created with bypass support. Postmortem examination showed a coronary artery fistula rising from the right ventricle and a complete absence of both coronary ostia. This rare finding has been reported sporadically in the English literature.